'At 31, my sister is one of the youngest people in Britain to get dementia'

Among the typical family portraits of birthdays, holidays and celebrations in the study of the Suffolk farmhouse belonging to Becky Barletta’s parents, is a landscape shot of the Himalayas, shrouded in mist.

It was taken by Becky in 2013 while on a six-week trek with her then boyfriend, Luca, in Nepal’s Annapurna Massif. Her uncle, James Dorrington, had just died aged 59, only a few years after he had been diagnosed with frontotemporal dementia, a rare and cruelly aggressive form of the disease, which had also just claimed the life of a cousin in her early 40s.

As Becky could not make it back for her uncle’s funeral, she instead decided to pay tribute by spelling out his name in rocks on the mountain slope: “James. Father. Brother. Son.”

Nobody then could have imagined that this bright, happy and supremely fit young woman with her whole life ahead of her would be the next to succumb. But, three years later, only a few months after her wedding day, Becky received the same terrible diagnosis.

At the time, she was 31, making her one of the youngest people in the country ever to be diagnosed with the disease. Her subsequent decline has been as rapid as it is heartbreaking.

“I have to remember not to talk about her in the past tense because she is still with us,” says her 30-year-old sister Sophie Gilbert. “It is horrendous to watch and I miss her so much. I haven’t seen a glimmer of the old Becky for ages now.”

Sophie lives next door to her parents in a converted barn with her husband Ben and two children, Alfie, two, and Emilia, who was born just three months ago. The former graduate from the London College of Fashion now works for the family bakery business, Dorrington’s, which runs 16 shops across Essex, Hertfordshire and Cambridge and marks its centenary in 2019.

Frontotemporal dementia – named after its destruction of lobes in the brain which control emotions and social behaviour – is a hereditary disease.

While the research remains scant, typically each of the children or siblings of someone with a genetic mutation that can cause the disease has a 50 per cent chance of carrying it themselves, although that does not necessarily mean they will then develop symptoms. Between 10 and 15 per cent of sufferers have several close relatives in different generations affected.

Since Becky’s diagnosis, the family have all been blood-tested, aside from Sophie’s children because of their age. But while typically the disease is inherited from a defect in three genes (MAPT, GRN or C9ORF72), Sophie says there is no common defective gene yet found shared between those in her family who have developed the disease. As a result, there is no definite test that can be done to reassure them.

FAQ | Pick’s Disease

In this, her only national newspaper interview, Sophie admits the family are only deciding to speak out in the hope of perhaps finding a cure to help others in the future.

“The thought is constantly there,” she says while cradling Emilia on her knee. “Not only to me, but my children, and I’ve got cousins who are all in the same boat. Every time I forget a word, I worry. Everybody may say that I won’t get it, but you just never know.”

Sophie and Becky were close growing up, and she recalls with a smile family holidays together to Walberswick on the Suffolk coast. After attending separate fee-paying sixth-form colleges in Cambridge, Sophie moved to London and Becky to Norwich to embark on an art foundation degree pursuing her love for photography.

When she graduated, Becky headed out to Val d’Isère to complete her first ever ski season. She worked as a chalet girl to pay her way, but over subsequent years took her exams to become a qualified ski instructor. “She loved the scenery, freedom and skill involved,” Sophie says. “She always wanted to be better.”

During a ski season in New Zealand in 2012, she met Luca, an Italian, and himself a ski instructor. Even when her dementia began to eat away at her speech and memories, Becky could still recall their first encounter. “Love at first sight” is how she remembered it.

It was, for a time, an idyllic life. During winters they would ski, and in summer run a hut Luca established for walkers in the Italian Alps. In 2014, while on holiday to Egypt, Luca proposed.

“She was so excited about getting married and had always dreamed of having three children,” Sophie says. “They were completely in love.”

In the months leading up to the wedding, the first symptoms of the disease began to show. Sometimes, she would glaze over and forget to say hello and became obsessed with watching YouTube videos on her phone.

“She was going a bit crazy with exercise,” Sophie says. “We thought it might just be the stress, or even some sort of anorexia. Then she would be fine for a while and we all just thought we were worrying about nothing. Nobody thought about dementia.”

They were married in October 2015. Sophie was the maid of honour, as her sister had been for her. A marquee for 160 guests was erected on the family farm, and the sun shone all day.

“She looked so beautiful in her dress,” Sophie says. “I’m just so thankful she had such an amazing wedding day.”

The month coincided with the 60th birthday of their mother, Caroline, and, rather than a honeymoon, the whole family decamped to Barbados. It was here where Becky’s symptoms began to suddenly worsen. She took little interest in activities or her young nephew Alfie, and at meal times would ignore food and drink a lot.

In early 2016, she was suspended from work and Sophie and her mother flew out to Verbier.

“That was when mum started seeing more of the signs that she had seen with her brother,” Sophie says. “She just panicked and said: ‘I think it might be this again.’ What it must be like for her just doesn’t bear thinking about.”

Eventually, they persuaded Becky to see the same doctor at Addenbrooke’s Hospital in Cambridge who had diagnosed her uncle. The results came back on August 24 last year. “It was the worst day of all of our lives,” Sophie says. “It was like a death in the family.”

Becky’s deterioration since then has been rapid. Her parents have recruited a full-time carer to help around the house, and Luca combines his work in Italy with travelling back each week to be with his wife. “It has been absolutely devastating for him,” Sophie says.

At the end of our conversation, Sophie takes me to meet her sister. She repeats one story endlessly about the family border terrier, Tiggy, and blows constant raspberries.

She is still fit and mobile, knows the names of her family and counts to 19 – her lucky number. Sometimes, however, when she sings, her songs turn to screams, and Sophie admits she cannot leave her alone with her children.

“You try and carry on as normal and then some days it just takes your breath away,” she says. “I’ve watched her walking up the garden path towards our house and she just doesn’t look like herself. It breaks my heart.”

Sophie Gilbert

A week on Sunday, the family are organising a walk around the farm to raise funds for the Alzheimer’s Society. They will be joined by wider relatives, friends and perhaps Becky herself.

Doctors fear she may only have a few years left, although Sophie admits the bereavement is already taking place.

“You just try and desperately cling on to bits of her,” Sophie says. “And then it’s gone before you know it and you can’t get it back.”

• To donate to Walking For Becky, in aid of the Alzheimer’s Society, go to bit.ly/beckyalzheimers

[https://www.justgiving.com/ fundraising/mw250123?utm_id=25 ]